Ko Jae Sung, Yi Nam-Joon, Suh Kyung Suk, Seo Jeong Kee
Department of Pediatrics Surgery, Seoul National University College of Medicine, Seoul, South Korea.
Pediatr Transplant. 2012 Mar;16(2):195-200. doi: 10.1111/j.1399-3046.2012.01661.x.
Fibropolycystic liver disease includes CHF, Caroli's syndrome, and Caroli's disease. Patients with Caroli's disease and Caroli's syndrome have an increased risk of recurrent cholangitis, intrahepatic calculi, biliary cirrhosis, and cholangiocarcinoma. The aim of this study was to examine the post-transplantation outcomes of children with fibropolycystic liver disease. Of the 158 children transplanted at Seoul National University Hospital, there were four patients with Caroli's syndrome, two patients with CHF, and one patient with Caroli's disease. One patient underwent combined liver/kidney transplantation. Associated renal manifestations included ARPKD in three children and nephronophthisis in one child. The indications for LT were recurrent cholangitis, decompensated cirrhosis, and refractory complications of portal hypertension. Both graft and patient survival rates were 100% at a median follow-up period of two yr after LT. Three children with growth failure achieved catch-up growth after LT. In three patients with ARPKD, mean serum creatinine levels increased from 0.53 mg/dL at the time of LT to 0.91 mg/dL at the last follow-up (p = 0.01). LT is an excellent option for children with complications from fibropolycystic liver disease. Renal function should be monitored cautiously after LT in the patients with ARPKD.
纤维多囊肝病包括先天性肝纤维化（CHF）、卡罗利综合征和卡罗利病。卡罗利病和卡罗利综合征患者发生复发性胆管炎、肝内结石、胆汁性肝硬化和胆管癌的风险增加。本研究的目的是检查纤维多囊肝病患儿的移植后结局。在首尔国立大学医院接受移植的158名儿童中，有4例卡罗利综合征患者、2例先天性肝纤维化患者和1例卡罗利病患者。1例患者接受了肝肾联合移植。相关的肾脏表现包括3例儿童患常染色体隐性多囊肾病（ARPKD）和1例儿童患肾单位肾痨。肝移植的指征为复发性胆管炎、失代偿性肝硬化和门静脉高压的难治性并发症。肝移植后中位随访期2年时，移植物和患者生存率均为100%。3例生长发育迟缓的儿童在肝移植后实现了追赶生长。在3例常染色体隐性多囊肾病患者中，平均血清肌酐水平从肝移植时的0.53mg/dL升至最后一次随访时的0.91mg/dL（p=0.01）。对于有纤维多囊肝病并发症的儿童，肝移植是一个很好的选择。对于常染色体隐性多囊肾病患者，肝移植后应谨慎监测肾功能。
